Lymphoma

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Recommendation Grade
AYAs presenting with hepatosplenomegaly should be referred immediately to a specialist.
C
Recommendation Grade
AYAs with a mediastinal or hilar mass on chest x-ray should be referred immediately to a specialist.
C
Recommendation Grade
AYAs should be referred urgently to a specialist if they have lymphadenopathy with one or more of the following, particularly if there is no evidence of local infection:
  • Non-tender, firm or hard lymph nodes
  • Lymph nodes greater than 2 cm in size
  • Progressively enlarging lymph nodes
  • Other features of general ill-health, fever or weight loss
  • Axillary node involvement (in the absence of local infection or dermatitis)
  • Supraclavicular node involvement.

AYAs should be referred immediately to a specialist if they have shortness of breath in association with the above signs; particularly if the shortness of breath is not responding to bronchodilators.

C

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Overview

Symptoms and signs

The majority of lymphomas (70-80%) arise from the lymph nodes; the rest develop outside the nodes.[1] Signs of lymphoma include adenopathy, mediastinal obstruction, abdominal mass, hepatomegaly, splenomegaly; common symptoms include fever, night sweats, weight loss, loss of appetite and enlarged lymph nodes (usually in the neck, under the arms or in the groin).[2][3] Tender lymph nodes are usually benign.[1] If glands in the chest are involved, shortness of breath or coughing may occur, while glands in the abdomen may cause bowel blockages.[3]

Hodgkin’s lymphoma typically presents with non-tender cervical and/or supraclavicular lymphadenopathy, cough, fever, night sweats and weight loss; symptoms often develop over a few months.[4][5]

Non-Hodgkin’s lymphoma can present with lymphadenopathy, breathlessness, superior vena-caval obstruction or abdominal distension and symptoms usually progress rapidly.[4]

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Clinical assessment

Adolescents and young adults (AYAs) presenting with hepatosplenomegaly (simultaneous enlargement of the liver and spleen) or a mass (mediastinal or hilar) on chest x-ray should be referred immediately to a specialist.

General practitioners (GPs) should refer AYAs presenting with lymphadenopathy and key symptoms of lymphoma (such as lymph nodes that are non-tender, firm or hard and/or progressively enlarging; and fever or weight loss) to a specialist urgently. Shortness of breath in association with these features, particularly if not responding to bronchodilators, is also an indication for urgent referral.[4]

Investigations such as FBC, x-ray and CT scan (where appropriate) may be initiated by the GP, but should not delay referral. Patients should be referred to specialists for biopsies or fine needle aspirates, if required.[1]

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Risk factors

Epstein Barr virus (EBV) infection is a strong risk factor for lymphoma;[6] family history of lymphoma or leukaemia has also been implicated.[1][3]

Infectious mononucleosis may increase the risk of Hodgkin lymphoma by two to three fold.[1] Non-Hodgkin’s lymphoma has been linked to immunosuppressive therapy and immunodeficiency syndromes (e.g. ataxia telangiectasia).[7]

However, lymphoma can occur in AYAs without any of these risk factors.

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References

  1. 1.0 1.1 1.2 1.3 1.4 Australian Cancer Network. Clinical practice guidelines for the diagnosis and management of lymphoma, a guide for general practitioners. Sydney: Cancer Council Australia and Australian Cancer Network; 2007.
  2. Bleyer A. CAUTION! Consider cancer: common symptoms and signs for early detection of cancer in young adults Semin Oncol 2009 Jun;36(3):207-12 [Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/19460578].
  3. 3.0 3.1 3.2 Adolescent and Young Adult Working Party of the Statewide Cancer Clinical Network. South Australian Adolescent and Young Adult Cancer Care Pathway: Optimising outcomes for all adolescent and young adult South Australians with a cancer diagnosis. Adelaide: South Australia Department of Health; 2010.
  4. 4.0 4.1 4.2 National Collaborating Centre for Primary Care. Referral Guidelines for Suspected Cancer. Clinical guideline 27. London: National Institute for Health and Clinical Excellence; 2005.
  5. Glass C. Role of the primary care physician in Hodgkin lymphoma Am Fam Physician 2008 Sep 1;78(5):615-22 [Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/18788239].
  6. Glaser SL, Lin RJ, Stewart SL, Ambinder RF, Jarrett RF, Brousset P, et al. Epstein-Barr virus-associated Hodgkin's disease: epidemiologic characteristics in international data Int J Cancer 1997 Feb 7;70(4):375-82 [Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/9033642].
  7. Linet MS, Wacholder S, Zahm SH. Interpreting epidemiologic research: lessons from studies of childhood cancer Pediatrics 2003 Jul;112(1 Pt 2):218-32 [Abstract available at http://www.ncbi.nlm.nih.gov/pubmed/12837914].

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